Bronchiectasis Presentation | Jindal Chest Clinic

"Bronchiectasis: A chronic lung disease marked by irreversible dilation and scarring of the airways."

1. Bronchiectasis

2. Bronchiectasis Definition Permanent destructive dilatation of the bronchi (following infection, destruction and fibrosis) Types: Cystic Cylindrical Localized or diffuse

3. Etiology of bronchiectasis Post-infectious , e.g. tuberculosis, pneumonia; childhood infection such as measles, mumps, whooping cough Connective tissue diseases, e.g. SLE, rheum arthritis , Sjögren’s syndrome, relapsing polychondritis Secondary to inhalation or aspiration, e.g . a foreign body Inflammatory bowel disease, e.g. ulcerative colitis Allergic bronchopulmonary aspergillosis Immune deficiency e.g. Secondary to ch lymphatic leukemia

4. Congenital causes of Bronchiectasis Cystic fibrosis Ciliary defects, e.g. primary ciliary dyskinesia, Young’s syndrome Kartagener’s syndrome Immune deficiency, e.g . IgA deficiency, X-linked agammaglobulinemia , Common variable immunodeficiency Congenital defects e.g. tracheobronchomegaly ( Mounier -Kuhn syndrome), pulmonary sequestration

5. Clinical Features Chronic cough and expectoration Sputum: Purulent/ muco -purulent, foul-smelling, large volume, thick and tenacious Haemoptysis , sometimes massive Recurrent exacerbations SIGNS: General malnutrition, pallor, edema Digital clubbing, osteoarthropathy Chest: Depends on site and extent of involvement If large, signs of lung volume reduction May be areas of bronchial breathing Coarse crepitations , Occasional rhonchi

6. Investigations General: Anemia, Hypoglobulinemia Chest radiography: CXR, CT scan (HRCT) Bronchography Sputum examination – For exacerbations. AFB to exclude TB, if suspected Smear for culture ECG, ECHO for cardiac evaluation in suspected chronic cor-pulmonale

7. Differential Diagnosis Pulmonary tuberculosis Cystic fibrosis COPD Allergic broncho -pulmonary aspergillosis Interstitial lung diseases Eosinophilic lung diseases Hypersensitivity pneumonias

8. Radiological features CXR: May appear normal in early, limited disease, left lower lobe hidden behind the heart in PA film. Thickened bronchial lines- tram lines Cystic shadows/ cavities with fluid levels HRCT : Almost diagnostic. Clear demonstration of site of involvement, Type of lesions, surrounding lung parenchyma, focal pneumonitis, areas of atelectasis. Clue to the underlying etiology ( eg ABPA)

9. Complications R ecurrent pneumonias Recurrent hemoptysis, sometimes massive Local lung destruction and cavitation Aspergilloma formation (fungal ball) in a cavity Metastatic spread Pulmonary hypertension and chronic cor pulmonale Chronic malnutrition Amyloidosis Chronic respiratory failure if extensive lung destruction and fibrosi

10. Management Bronchial hygiene: Postural drainage, Chest physiotherapy Antibiotics for infections Expectorant and mucolytics Management of complications, e.g hemoptyis , pulmonary hypertension (Chronic cor pulmonale ), respiratory failure Nutritional supplementation Surgical management: Resection, if localized Management of hemoptysis Lung transplantation ?

11. Recommendation for antibiotics use Bacterial infection First choice Second line Haemophilus influenzae Doxycycline , or Moraxella catarrhalis Co- amoxiclav ciprofloxacin Streptococcus pneumoniae Amoxicillin Clarithromycin MRSA Rifampicin and Rifampicin and trimethoprim doxycycline or or IV vancomycin linezolid or teicoplanin Ps aeruginosa Ciprofloxacin Ceftazidime and tobramycin or colistin

12. Prevention of infections Preventive vaccinations Bronchial hygiene measures: - Chest physiotherapy - Nebulization / steam inhalation - Respiratory muscle exercises Long term antibiotic use - Oral Nebulized

13. Kartagener’s Syndrome Ciliary dyskinesia i.e. abnormal ciliary movements Genetic abnormality Clinical features: Bronchiectasis Situs inversus , dextrocardia Chronic sinusitis Infertility

14. Allergic Broncho Pulmonary Aspergillosis Hypersensitivity to aspergillus in the tracheo -bronchial tree in patients with chronic asthma. Clinical Features: Severe attacks, sputum production; hard brown plugs; hemoptysis Radiology: CXR and HRCT: Fleeting opacities, typical patterns; bronchiectasis – proximal bronchi Diagnosis: Skin test: Immediate & delayed + ve Sputum for aspergillus + ve Serology + ve ; Total & Asperg specific IgE levels Treatment: Anti-inflammatory drugs (steroids), Anti- biotics , anti- fungals

15. Cystic Fibrosis A common condition in Caucasians – 1 in 2500 live births Genetic anomaly: Autosomal recessive mutation on chromosome 7; leads to protein Cystic Fibrosis Transmembrane Regulator, CFTR) abnormality Clinical Features: Multi-organ problem Bronchiectasis – thick viscid sputum Pancreatic insufficiency - diarrhoea Liver disease – biliary cirrhosis Sweat glands function abnormality Infertility Low bone mass

16. Cystic Fibrosis- Diagnosis Clinical features – Failure to thrive Intestinal obstruction Adults: Respiratory infections Radiological investigations, CXR, HRCT scans etc Positive sweat Test – High sweat chloride & Na levels on pilocarpine stimulation Gene analysis – demonstration of CFTR mutations

17. Cystic Fibrosis- Treatment Treatment of respiratory infection with antibiotics: Anti-pseudomonas cover Reduce sputum viscosity- mucolytics Improve airway clearance Management of pancreatic insufficiency Correction of malnutrition – high calorie, high fat diet; supplemental vitamins Gene therapy Lung transplantation

18. TUBERCULOSIS History Epidemiology Introduction

19. What is TB ? Infection caused by Mycobacterium tuberculosis ( Mtb ) i.e. Tubercle bacillus ( T.b .) Airborne – spreads by aerosols; enters the lungs through inhalation

20. HISTORY Ancient disease since BC era Also known as Consumption Wasting Phthisis “ Yakshma ” King’s evil Kochs ’ disease

21. TB in Antiquity Clear evidence of spinal TB Early Dynastic period (c.3400 BC) Egypt : Destruction and collapse of thoracic vertebrae with psoas abscess in the well preserved mummy of a member ( Nesperehan ) of 21 st Dynasty priesthood of Amin. Cave, 1939 Chinese Civilization Lung fever and Lung cough (Chinese writings – 2698 BC). Symptom of emaciation, cough, expectoration of blood and pus; cure was difficult; bizarre remedies – dung of animals & man, the urine of women and infants, the lungs of the hog and the ashes of hair. Hall, 1936

22. Babylon civilization Mention of TB 1948 and 1905 BC: Code of Hammurabi “His wife who is afflicted with the disease he shall not put away. She shall remain in the house which she has built and he shall maintain her as long as she lives.” Indo Aryans ‘A consumptive who is evidently master of himself, who has a good digestion, is not emaciated and is at the beginning of the disease the physician can cure’ and ‘the physician who wants great fame cures a man attacked by consumption’. Webb, 1936

23. TB in ancient India ( Rajyakshma ) Rig Veda (1500 BC) Ayur -Veda (700 BC) “… a consumptive … at the beginning of disease the physician can cure …” Laws of Manu (1000 BC) “… sufferers from TB are unclean …” Webb GB 1936; Brown L 1941; Keers RY 1978

24. Historical landmarks Tubercle bacillus (Mycobacterium tuberculosis): Discovered on March 24, 1882 by Robert Koch (Awarded Nobel Prize in 1905) Discovery of X-Ray (Wilhelm Roentgen, 1895) Bacillus Calmette Guerin (BCG) Chemotherapy: Streptomycin (1944), P.A.S., Isoniazid (1952) Ethambutal , Rifampicin Other new drugs Regimens and Strategies

25. Koch’s postulates The organism should be found in each case of the disease It should not be found in other diseases It should be isolated It should be cultured It should, when inoculated, produce the same disease It should be recovered from the inoculated animal

26. Epidemiology Incidence vs Prevalence Risk factors Disease burden Morbidity and mortality Global health challenge Higher incidence in low income countries India accounts for about 30% of global cases

27. TB is the leading single infectious cause of death in South-East Asia Number of deaths (1000s) Deaths from infectious agents in South-East Asia Tuberculosis HIV STD Malaria Tropical Diseases Measles

28. TB is the leading single infectious cause of death in India

29. TB is a Leading Killer of Women Deaths among women

30. Tuberculosis A Global Emergency TB kills 5,000 people a day – 2 million each year One third of the world’s population is infected with TB More than 100,000 children will die needlessly from TB this year Hundreds of thousands of children will become TB orphans this year HIV and MDRTB will make the TB epidemic much more severe unless urgent action is taken

31. TB Epidemiology in India TB infection ( Mx + ve ) 40% Prevalence (sputum + ve ) 0.23% Mortality 0.04% Overall prevalence 0.51%

32. Burden of TB in India 2 million new patients per year Over 450,000 deaths from TB annually TB kills more woman than all other causes of maternal mortality combined More than 100,000 women rejected (due to TB) More than 300,000 children leave schools to work as a result of parental TB Annual cost of disease – Rs. 12,000 crores Annual direct costs – Rs. 30 crores Productive work days lost – 100 million per year

33. Risk Factors Immuno -deficiency states HIV infections Patients with malignancies, leukaemias , lymphomas Patients on immuno -suppressant drugs (e.g. steroids) Malnutrition, drug-users, psychiatric disorders 3. Close contacts of patients Infants of sputum + ve mothers

34. 4. Poverty; living in crowded, slum areas; poorly ventilated houses 5. Alcoholism 6. Tobacco smoking 7. Patients with other diseases (comorbidities) - Diabetes - Hypothyroidism - Silicosis ( silico -tuberculosis) 8. Post-operative – gastrectomy

35. HIV Infection & TB Risk Annual risk – about 10% Life time risk of TB w.r.t . HIV - Negative 5-10% - Positive 50 % COINFECTION (HIV & TB) App. 1/3 of 20 million HIV pts.

36. TB and AIDS Lifetime Risk of TB

37. Homeostasis – unbalanced in infections Host defenses Pathogens

38. TB – An Infection Tubercle bacillus ( T.b ) Mycobacterium tuberculosis (MTB) Airborne – spreads by aerosols; enters the lungs through inhalation Interplay between the bacillus and the host defences Establishment of infection – Lesions in the lungs / lymph nodes / GIT/ other organs Spread to other sites/ organs Complications and Sequelae

39. Sequence of TB infection Inhalation of Mtb – localization in tracheo -bronchial tree Recruitment of macrophages and lymphocytes. Macrophages transform as Langhan’s cells Engulfed by alveolar macrophages ( defence cells) – either get killed or destroy the cells to penetrate alveolar walls and enter the lymphatics / blood vessels, reach regional LN

40. Langhan’s cells and lymphocytes form granulomas (Primary lesion) Primary lesion and regional lymphatics and LN together called Primary Complex (of Ranke) Fibrous capsule formation; may lie dormant in the LN (Latent TB) or spread through lymphatics / blood stream to bones, liver, spleen, GIT etc. Impart delayed type, cell-mediated immunity (demonstrated by Tuberculin or Mantoux test)

41. Bacillary multiplication 1 Bacillus 20 hrs 2 bacilli 10 days 5000 (Nodule) 1 month > 1 billion (large cavity)

42. Natural history of untreated Primary TB Time from Infection TB involvement 3-8 wks Primary complex 3-6 mths Meningeal , miliary,pleural Up to 3 yrs GIT, Bones & joints, LNs About 8 yrs Renal tract 3 yrs onwards Post primary disease

43. Continuing Infection One Sputum positive (untreated, undetected) Infects 6-12 persons in 1 st year upto 24 in 2 year life span

44. The National Problem Large pool of patients Renewed and perpetuated Difficult to approach Difficult to find, hold and treat Shortage of beds

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